| Objective:To improve the recognition of IgG4-related disease by summarizing clinical manifestations,pathological features,treatment and prognosis of 32 patients with IgG4-related disease.Methodology:The patients were out-patients,or were admitted to Jinling hospital between 2012 and 2017 according to the criteria for IgG4-RD.The clinical manifestations,pathology,treatment and prognosis of IgG4-RD patients were analyzed retrospectively.Disease activity and damage were assessed by the IgG4-RD Responder Index(RI).Results:There were 26 male and 6 female with an average age of 57.3±13.8 years old.All patients met the existing IgG4-RD diagnostic criteria and 21 cases were confirmed by pathology review.The most common affected organs are the kidney(17/32),the pancreas(7/32),the submandibular gland(8/31),etc.Clinical manifestations vary,20/32 cases have organs swelling,and 9/32 cases with allergic diseases such as drug allergy or asthma history.Serum IgG4 increased in 29/29,peripheral blood eosinophils in 8/32,globulin in 21/32,IgG in 16/22,IgE in 17/22,and low serum C3 and C4 levels in 8/22.There were 17 cases with renal involvement,including 2 cases of nephrotic syndrome,13 cases of urinary abnormalities,8 cases of acute kidney injury,3 cases of chronic renal falure,and 4 cases of renal enlargement.Compared with the group without renal involvement,there was more severe anemia(107.1±24.3g/L vs 125.3±15.6g/L,P=0.016),higher RI[7(6,9)vs 6(6,6),p=0.008],higher serum creatinine levels are[1.75(0.95,3.42)mg/dl vs 0.67(0.55,0.87)mg/dl,p=0.01],higher proportion with elevated blood ?-macroglobulin(7/8 vs 0/4,p=0.010),lower eGFR(52.3±42.3ml/min/ml vs 105.5±17.1ml/min/ml,P=0.00)in renal involvement group.At the same time,we observed that the proportion of low-complement in the renal involvement group was higher than that in non-renal involvement group(7/14 vs 1/8).There were 14 patients with normal complement and 8 patients with low complement.The IgG4 level was higher(28097.5±22652.3mg/L vs 7180±4454.7mg/L,P=0.035),and IgG level was also higher(37.8±19.2g/l vs 22.0±12.8g/l,p=0.043)in the low-complement group.12 patients underwent renal biopsy,the histological diagnosis was IgG4-related tubulointerstitial nephritis(TIN)in 10 cases,membranous glomerulonephritis in 2.It is characterized by infiltration of lymphoplasmacytic cells,and eosinophilic infiltration and tracheitis can be found in some cases,with varying degrees of interstitial fibrosis.15 cases with renal involvement treated with glucocorticoid or immunosuppressive agents all showed showed improvement in urine test or renal function.One patient with renal replacement therapy got rid of dialysis,and most patients had lower IgG4 level after treatment.Renal function and urine test was progressing in one patient rejected glucocorticoid therapy.Conclusion:IgG4-RD is a multi-systemic inflammatory disease.The clinical manifestations vary depending on the affected organs,and there is no specific biomarker.Diagnosis should be considered for patients with organ enlargement or organ dysfunction combined with high immunoglobulin.Increased eosinophilic granulocytes,elevated IgE,or low-complement may contribute to disease diagnosis.Renal involvement is characterized by renal tubular interstitial lesions,or associated with membranous lesions,then IgG4-RKD may be considered.The disease responds well to glucocorticoid or immunosuppressive therapy. |
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